Journal of Blood Medicine最新文献

Purpose: Prophylaxis with recombinant factor VIII (rFVIII) products is the gold-standard treatment for hemophilia A. However, interindividual differences affect the half-life and clearance of rFVIII products. The myPKFiT is a web-based medical-device software program for population pharmacokinetic (PK) simulation of FVIII products to guide accurate FVIII doses and dosing intervals. In this Japanese multicenter observational study, the efficacy of regimen adjustment using myPKFiT was examined.

Patients and methods: Male patients with hemophilia A undergoing personalized treatment with myPKFiT using either octocog alfa or rurioctocog alfa pegol were included. Patients were aged <18 years. Primary endpoint was annualized bleeding rate (ABR). Secondary endpoints were ABR by type of bleeding, rFVIII product consumption, physical activity level, quality of life, and frequency of rFVIII administrations. Results are presented descriptively; however, for exploratory analysis, data before and after regimen adjustment were compared using the Wilcoxon signed-rank test.

Results: Seven patients aged 3-17 years (median age 13 years) participated in the study. Mean ABR for all bleeds decreased by 0.86 after PK-guided regimen adjustment. Four patients showed zero ABR before and after regimen adjustment using myPKFiT. No significant differences were noted in the consumption of rFVIII products. However, mean rFVIII consumption decreased in two patients after PK-guided regimen adjustment. Three patients increased physical activity and, according to the treatment based on the PK-guided regimen adjustment, this resulted in no increased bleeding.

Conclusion: The results from this study in a small number of patients suggest that PK-guided regimen adjustment with myPKFiT may support optimization of the individual prophylactic administration of the FVIII products octocog alfa and rurioctocog alfa pegol.

Study registration: UMIN000044800.

Purpose: To study the platelet adhesion and aggregation behaviour of late pregnancy women under arterial shear rate using microfluidic chip technology and evaluate the risk of thrombosis in late pregnancy.

Methods: We included pregnant women who were registered in the obstetrics department of our hospital between January 2021 and October 2022 and underwent regular prenatal examinations. Blood samples were collected at 32-35 weeks of gestation for routine blood tests and progesterone, oestradiol, and platelet aggregation function. A microfluidic chip was used to construct an in vitro stenosis vascular model to explore the platelet reactivity at shear rates of 1000s-1, 1500s-1 and 4000s-1. Flow cytometry was used to analyse the effect of shear rate induction on the expression of platelet membrane surface fibrin receptor (PAC-1) and P-selectin (CD62P) in pregnant women.

Results: Compared to the non-pregnant healthy control group, the white blood cell count increased and platelet count decreased significantly in late pregnant women (P < 0.05), and platelet reactivity to agonists increased under non-flow conditions (adhesion and aggregation rates, P < 0.05). Microfluidic chip technology showed that platelet aggregation in late pregnant women increased significantly (P < 0.05) in the shear-rate environment and was positively correlated with the shear rate. The degree of aggregation at 4000s^-1 was more evident, but the stability of platelet aggregates was low. Shear rate increased PAC-1 and CD62P expression.

Conclusion: Microfluidic chip technology was used to analyse the platelet aggregation function under arterial shear rate combined with flow cytometry to detect platelet activation, which was consistent with the traditional non-flow conditions used to evaluate platelet function. However, microfluidic technology can simulate a more realistic in vivo shear rate environment, providing more effective clinical application data and a theoretical basis for the diagnosis and prevention of platelet dysfunction and thrombotic diseases during pregnancy.

Systemic sclerosis (SSc) is a multi-system disease characterized by a dysregulated immune system. Autologous hematopoietic cell transplantation (AHCT) is the only treatment that has been shown to confer significant benefit in controlling disease and improving survival for patients with SSc. A diagnosis of multiple myeloma (MM) after the diagnosis of SSc is rare and optimal treatment in such cases remains unclear. We here report a case of a female patient who was diagnosed with MM while she was undergoing evaluation for AHCT due to SSc. A novel conditioning regimen for AHCT, with therapeutic efficacy in SSc and MM was offered to the patient, resulting in long term remission of both diseases.

Previous research has unveiled an intriguing positive association between the AB blood group and mental disorders in general. In this study, we compared ABO blood groups with five major groups of mental disorders to attain a higher level of specificity. The analyses were conducted using data from the CoLaus|PsyCoLaus study (N=5111). They revealed that the AB blood group exhibited a positive association with both neurodevelopmental disorders (RR 2.29, CI 1.38-3.82) and substance use disorders (RR 2.25, CI 1.38-3.65) after adjusting for sex and childhood adversities. These associations could be replicated with respect to the familial aggregation of neurodevelopmental and substance use disorders. Large databases are needed to achieve more detailed results related to specific disorders.

Purpose: Trauma-associated coagulopathy has been considered to develop as a result of increased fibrinolysis due to massive bleeding, tissue damage and hypoperfusion. However, it has not been investigated whether hematoma may cause trauma-associated coagulopathy. Using experimental animal model, we analyzed the effects of hematoma formation on coagulation and fibrinolysis parameters.

Materials: Male Wistar rats were used for the studies.

Methods: We made an animal model of subcutaneous hematoma without tissue injuries. This model can be categorised as a kind of trauma models. We created experimentally subcutaneous hematomas in test animals using blood collected from other animals. We performed blood sampling to measure blood cell counts and coagulation parameters from test animals at 1, 6, 24, 48 and 96 hours after hematoma generation. Blood samples were collected and immediately sent for measurement of CBC, Prothrombin, FDP, D-dimer, Fibrinogen, Antithrombin, AST and ALT. Furthermore, after 1, 24 and 48 hours, we performed dynamic evaluation of coagulation/fibrinolysis function using thromboelastometry method.

Results: After the hematoma were created, FDP and D-dimer increased over time, and reached a plateau after 48 hours. During the period, there was no decrease in Fibrinogen and Antithrombin, and no thrombocytopenia occurred. Moreover, no obvious changes in coagulation/fibrinolysis function were observed employing thromboelastometry.

Discussion: Elevated FDP and D-dimer after hematoma creation are assumed to be synthesized in the hematoma, not in the streaming blood. Thromboelastometry also shows that elevated levels of FDP and D-dimer are not caused by intravascular coagulation and subsequent fibrinolysis.

Conclusion: The study showed that subcutaneous hematomas caused increases in FDP and D-dimer levels, without activating the blood coagulation/fibrinolysis system.

Background: Elevated vitamin B12 (B12) levels are linked to an increased risk of cancers, including hematological malignancies. This study focuses on the relationship between elevated B12 and myeloproliferative neoplasms (MPNs): Polycythemia Vera (PV), Primary Myelofibrosis (MF), Essential Thrombocytosis (ET), and Chronic Myeloid Leukemia (CML). Elevated B12 in MPNs is believed to arise from increased transcobalamin I (TCI) secretion by proliferating leukocytes, leading to higher serum levels. B12 may serve as a diagnostic and prognostic biomarker for these conditions. However, its sensitivity, specificity, and cutoff levels are unclear.

Aim: To assess the prevalence of high B12 levels in MPN patients, determine the median levels, identify a diagnostic cutoff, and evaluate the sensitivity and specificity of B12 as a marker.

Methods: Data were retrieved from the National Center for Cancer Care and Research in Doha, Qatar, for MPN patients from January 2016 to December 2022.

Results: A total of 467 patients were included: 232 with CML, 98 with PV, 88 with ET, and 50 with MF. The majority were male (66%) and of Asian origin (56%), with a median age of 48.7 years. CBC results showed median hemoglobin of 9.2 g/dL, WBC count of 73 x 10^3/uL, and platelet count of 531 x 10^3/uL. Elevated B12 levels were found in 95 patients (20%): 71% CML, 14% PV, 10% MF, and 5% ET. Extreme elevations were seen in 59 patients. The mean B12 level decreased from 747.3 ± 686.5 pg/mL before treatment to 397.9 ± 343.7 pg/mL after one year (p=0.01). Median levels were 458 pg/mL (718) before treatment and 301 pg/mL (229) after. In the extreme high B12 group, the mean was 1722 pg/mL before and 677 pg/mL after treatment.

Conclusion: Elevated B12 levels are associated with disease activity in CML. However, their role as a reliable marker for disease monitoring remains uncertain, and further studies are needed to confirm their utility for CML progression.

Purpose: Atrial fibrillation (AF) being a prevalent cardiovascular condition globally, has an increased risk of stroke and other complications. The effective management of AF often involves the use of oral anticoagulants (OACs) to prevent thromboembolic events. This study aimed to evaluate anticoagulation knowledge and medication adherence in AF patients on OACs at a tertiary care center in Nepal.

Patients and methods: A descriptive cross-sectional study involving patients diagnosed with AF who were prescribed OACs at the Cardiology Department of Dhulikhel Hospital, Nepal, was conducted from March to June 2024. Data were collected using questionnaires, including the Oral Anticoagulation Knowledge Tool (AKT) and the Adherence in Chronic Diseases Scale (ACDS). The study included patients from the Dhulikhel Atrial Fibrillation (DAF) Registry database along with other AF patients visiting the cardiac department. Descriptive statistics were used to summarize patient demographics, knowledge scores, and adherence levels. Inferential statistics were used to observe the associations.

Results: Among the 114 AF patients enrolled in the study, 93 were receiving OAC therapy and were interviewed. The mean age of the participants was 66.84 ± 12.3 years, with the majority being female (57%). The study revealed that a significant portion of patients lacked adequate knowledge about their OAC therapy, with only 48% having adequate knowledge as per the AKT. Additionally, 83.9% of the patients demonstrated high adherence to their medication regimen, whereas 16.1% showed medium adherence. The duration of use of OACs was found to be significantly associated with adequate anticoagulation knowledge.

Conclusion: The study findings indicate that a significant proportion of AF patients in Nepal lack adequate anticoagulation knowledge, highlighting an opportunity for improved educational interventions.

Gallbladder adenocarcinoma has a high mortality rate, with approximately 1.7% cancer-related deaths worldwide. Cancer-associated thrombosis (CAT), including deep vein thrombosis (DVT), can significantly increase the risk of mortality within cancer patients, especially in pancreatic, brain, and intra-abdominal cancers, as well as in advanced and metastatic cancers. In this case report, there was a 45-year-old male patient diagnosed with advanced gallbladder adenocarcinoma UICC stage IVB with a TNM stage of T2b, N0, M1 with liver metastases who experienced pain and swelling in both lower limbs after undergoing a VI-A cycle of chemotherapy with gemcitabine capecitabine. The risk of thrombosis was calculated using the modified Khorana-Vienna CAT scores, which increased during every chemotherapy session. In this case, the Khorana-Vienna CAT score was calculated during two latest cycle of chemotherapy that somewhat considered delayed as the patient had already shown hypercoagulopathy symptoms and developed a poorer prognosis. Early CAT scoring, ideally before starting chemotherapy session, potentially improves thrombosis prognosis. The patient's condition improved after administration of antithrombotic agents. Chemotherapy agents and other factors, including the cancer site and presence of metastatic cancer, influence the risk of CAT. Risk predictor scores are required to assess the risk of CAT and benefits of prophylactic treatment. Prophylactic therapy can be initiated in patients with high-risk CAT, calculated using the modified Khorana and Vienna CAT scores, to prevent thrombosis and improve patient outcomes.

Background: Complete blood counts (CBC) are commonly used in diagnostic medicine to evaluate normal and abnormal hematological status. Furthermore, reference values (RVs) of CBC supplied by researchers are the most reliable means of the judgment-making stage and can aid interpretation and accurate diagnosis of diseases. Reference values vary between peoples because of differences in lifestyle, dietary habits, ethnicity and environment. Moreover, the Clinical and International Standards Institute (CISI) advises determining the RVs for each area. There are no RVs for CBC in Yemen. Therefore, this study aimed to determine the common RVs of CBC for healthy adults in Ibb City in the middle of Yemen.

Methods: A cross-sectional study was conducted from April 1, to November 30, 2023. Of the 623 adults who participated in this study, 433 (aged 18-80 years) were included in the final analysis after applying exclusion criteria. The mean, median, and 95^th percentile RVs (2.5^th-97.5^th percentiles) were calculated for gender, age, and residence by the GraphPad Prism 8.0.1.

Results: The RVs of hemoglobin (Hb) 11.16-17.54g/dl, red blood cells (RBC) 3.890-6.340×10¹²/l, hematocrit (HCT) 33.03-49.30%), mean corpuscular volume (MCV) 72.83-94.55fl), mean corpuscular hemoglobin (MCH) 23.95-33.55pg, mean corpuscular hemoglobin concentration (MCHC) 32.97-36.7354g/dl, platelet (PLT) count 140.0-418.6×10⁹/l, total white blood cells (WBC) 2.810-8.797 ×10⁹/l and WBC differential count (basophils 0.000-1.000%, neutrophils 30.10-69.17%, eosinophils 1.500-5.000%, lymphocytes 23.86-63.45% and monocytes1.873-5.600%). Significantly higher median values were observed in males compared to females for Hb (P<0.0001), RBC (P<0.0001), HCT (P<0.0001), lymphocyte (P=0.0197) and monocytes (P=0.0009). Contrariwise, females demonstrated significantly higher neutrophils (P=0.0009), eosinophils (P=0.0020), basophils (P<0.0001) and platelets (P=0.0324) than males. This study showed differences in the RVs of CBC compared to those reported in other countries in the Middle East, Asia, Africa, and Europe.

Conclusion: In this study, the reference values of CBC are considered as a benchmark that may assist in accurately judging laboratory results and enhancing medical and clinical services for adults in Ibb City, Yemen.

Background: Venous thromboembolism is the second leading cause of mortality among cancer patients. The Khorana Risk Assessment Score (KRAS) is widely acknowledged as the most validated tool in this context.

Aim: To assess the thrombotic risk in cancer patients using the modified Khorana Risk Assessment Score, examine the association between modified KRAS and soluble P-selectin levels, and document the utilization of thromboprophylaxis among cancer patients at the University of Calabar Teaching Hospital.

Methods: This was a cross-sectional hospital-based recruiting 100 cancer patients. Seven millilitres of blood were collected for complete blood count and P-selectin assay. Continuous variables were expressed as mean and standard deviation, while categorical variables were summarized using frequencies. Chi-square was employed to compare VTE risk status across genders, different cancer types, and guideline compliance. The significance level was set at 0.05.

Results: Participants age ranged from 19 to 87 years, with a male-to-female ratio of 1:1.6. The most common female cancer was Breast at 40.32% and prostate cancer at 65.79% was the most common in males. Seventy nine percent and 21% of participants had intermediate and high-risk modified KRAS scores respectively. The median level of soluble P-selectin among cancer patients was 23.00 within the interquartile range. Significant associations were observed between cancer types and sex, VTE risk assessment and cancer types, and cancer types and risk score.

Conclusion: The risk of VTE among cancer patients ranges from intermediate to high, going by the modified Khorana risk score irrespective of the P selectin level, with underutilization of thromboprophylaxis. There is little adherence to the Khorana score in our setting, hence the need for greater application and knowledge of this predictive score in clinical practice to improve outcomes and quality of life.